Department of Biology and Biochemistry, University of Bath, Bath, UKBA2 7AY.
Metallomics. 2011 Mar;3(3):229-38. doi: 10.1039/c0mt00047g. Epub 2010 Oct 29.
The prion protein is well known because of its association with prion diseases. These diseases, which include variant CJD, are unusual because they are neurodegenerative diseases that can be transferred between individuals experimentally. The prion protein is also widely known as a copper binding protein. The binding of copper to the prion protein is possibly necessary for its normal cellular function. The prion protein has also been suggested to bind other metals, and among these, manganese. Despite over ten years of research on manganese and prion disease, this interaction has often been dismissed or at best seen as a poor cousin to the involvement of copper. However, recent data has shown that manganese could stabilise prions in the environment and that chelation therapy specifically aimed at manganese can extend the life of animals with prion disease. This article reviews the evidence for a link between prions and manganese.
朊病毒蛋白因与朊病毒疾病有关而广为人知。这些疾病包括变异型克雅氏病，它们不同寻常，因为它们是神经退行性疾病，可以在实验中在个体之间传播。朊病毒蛋白也被广泛认为是一种铜结合蛋白。铜与朊病毒蛋白的结合可能是其正常细胞功能所必需的。朊病毒蛋白还被认为可以结合其他金属，其中包括锰。尽管对锰和朊病毒疾病的研究已经进行了十多年，但这种相互作用经常被忽视，或者充其量被视为铜参与的一个次要因素。然而，最近的数据表明，锰可以使环境中的朊病毒稳定下来，并且专门针对锰的螯合疗法可以延长患有朊病毒疾病的动物的寿命。本文综述了朊病毒和锰之间联系的证据。
